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Juvenile systemic lupus erythematosus

Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
September 28, 2016: Revista Brasileira de Reumatologia
Wildéa Lice de Carvalho Jennings Pereira, Edna Maria Vissoci Reiche, Ana Paula Kallaur, Sayonara Rangel Oliveira, Andréa Name Colado Simão, Marcell Alysson Batisti Lozovoy, Lucas José Vaz Schiavão, Paula Raquel do Vale Pascoal Rodrigues, Daniela Frizon Alfieri, Tamires Flauzino, Damacio Ramón Kaimen-Maciel
OBJECTIVE: The aim of this study was to report the frequency of autoimmune disorders and autoantibodies in 22 patients with neuromyelitis optica (NMO), as well as whether the seropositivity for autoantibodies differs between anti-aquaporin 4 (AQP4) positive and AQP4 negative NMO patients. METHODS: Demographic, medical records, and a profile of autoantibodies were evaluated in 22 NMO patients, including AQP4, anti-thyroid-stimulating hormone receptor, antinuclear antibodies (ANA), anti-thyroperoxidase (anti-TPO), anti-thyroglobulin (anti-Tg), anti-double-stranded DNA, anti-neutrophil cytoplasmic, anti-cyclic citrullinate peptide, rheumatoid factor, anti-SSA/Ro, anti-SSB/La, anti-Smith antibodies (anti-Sm), anti-ribonucleoprotein, anti-nucleosome, and anti-Scl70...
October 3, 2016: Acta Neuropsychiatrica
Ana Paula Sakamoto, Clovis Artur Silva, Mariana Paes Leme Ferriani, Rosa Maria Rodrigues Pereira, Eloisa Bonfá, Claudia Saad-Magalhães, Eunice Okuda, Simone Appenzeller, Francisco Hugo Gomes, Ana Luiza Garcia Cunha, Mirna Henriques Tomich Salume, Daniela Petry Piotto, Maria Teresa Terreri
Chronic arthritis (CA) is an unusual condition in childhood-onset systemic lupus erythematosus (cSLE) and data in children is very limited. The aim of the study is to assess CA in a large population of cSLE patients, in a multicenter cross-sectional study including 852 cSLE patients followed in ten Pediatric Rheumatology referral services in state of São Paulo, Brazil. CA was observed in 32/852 (3.7 %) cSLE patients mostly in hands and ankles. Chronic monoarthritis was diagnosed in four cSLE patients, oligoarthritis in nine and polyarthritis in 19...
September 14, 2016: Rheumatology International
Christelle Domngang Noche, Giles Kagmeni, Viola Dohvoma, Assumpta Lucienne Bella, Come Ebana Mvogo, Madeleine Singwe-Ngandeu
PURPOSE: To determine the profile of ophthalmic manifestations in chronic inflammatory rheumatic diseases (CIRD). METHODS: Observational study at the Yaounde Central Hospital and Innel Medical Centre (2004 to 2012). RESULTS: The study population (n = 36) consisted of 14 men and 22 women with average age of 47.9 ± 17.2 years. Cases of CIRD were rheumatoid arthritis (n = 16), systemic lupus erythematosus (n = 8), ankylosing spondylitis (n = 8), mixed connective tissue disease (n = 2), scleroderma (n = 1), and juvenile idiopathic arthritis (n = 1)...
September 6, 2016: Ocular Immunology and Inflammation
Eve Mary Dorothy Smith, Andrea Lyn Jorgensen, Angela Midgley, Louise Oni, Beatrice Goilav, Chaim Putterman, Dawn Wahezi, Tamar Rubinstein, Diana Ekdawy, Rachel Corkhill, Caroline Ann Jones, Stephen David Marks, Paul Newland, Clarissa Pilkington, Kjell Tullus, Michael William Beresford
BACKGROUND: Conventional markers of juvenile-onset systemic lupus erythematosus (JSLE) disease activity fail to adequately identify lupus nephritis (LN). While individual novel urine biomarkers are good at detecting LN flares, biomarker panels may improve diagnostic accuracy. The aim of this study was to assess the performance of a biomarker panel to identify active LN in two international JSLE cohorts. METHODS: Novel urinary biomarkers, namely vascular cell adhesion molecule-1 (VCAM-1), monocyte chemoattractant protein 1 (MCP-1), lipocalin-like prostaglandin D synthase (LPGDS), transferrin (TF), ceruloplasmin, alpha-1-acid glycoprotein (AGP) and neutrophil gelatinase-associated lipocalin (NGAL), were quantified in a cross-sectional study that included participants of the UK JSLE Cohort Study (Cohort 1) and validated within the Einstein Lupus Cohort (Cohort 2)...
September 3, 2016: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
M Al-Obaidi, D Saunders, S Brown, L Ramsden, N Martin, E Moraitis, C A Pilkington, P A Brogan, D Eleftheriou
The aim of this study was to describe the abnormalities identified with conventional MRI in children with neuropsychiatric systemic lupus erythematosus (NPSLE). This was single-centre (Great Ormond Street Hospital, London) retrospective case series of patients with juvenile NPSLE seen in 2003-2013. Brain MR images of the first episode of active NPSLE were reviewed. All patients fulfilled the 1999 ACR case definitions for NPSLE syndromes. Presenting neuropsychiatric manifestations, immunological findings and treatment are reported...
October 2016: Clinical Rheumatology
Cecilia Beatrice Chighizola, Voon H Ong, Pier Luigi Meroni
Cyclosporine A, an inhibitor of calcineurin, exerts an immunomodulator action interfering with T cell activation. Even though novel therapeutic tools have emerged, CyA still represents a suitable option in several clinical rheumatology settings. This is the case of refractory nephritis and cytopenias associated with systemic lupus erythematosus. Furthermore, CyA is a valued therapeutic tool in the management of uveitis and thrombophlebitis in course of Behçet's disease. Topical CyA has been proven to be beneficial in the dry eye of Sjogren's syndrome, whereas oral treatment with CyA can be considered for the severe complications of adult onset Still's disease...
August 11, 2016: Clinical Reviews in Allergy & Immunology
Selin Aytaç, Ezgi Deniz Batu, Şule Ünal, Yelda Bilginer, Mualla Çetin, Murat Tuncer, Fatma Gümrük, Seza Özen
Macrophage activation syndrome (MAS) is a hyper-inflammatory disorder secondary to a rheumatic disease such as systemic juvenile idiopathic arthritis (SJIA) and systemic lupus erythematosus (SLE). We aimed to present the characteristics of our pediatric MAS patients. Clinical features, laboratory parameters, treatment, and outcome of 34 patients (28 SJIA; six SLE; 37 MAS episodes) followed at a tertiary health center between 2009 and 2015 were retrospectively reviewed. The median age at MAS onset was 11 years...
October 2016: Rheumatology International
Benito Lourenço, Katia T Kozu, Gabriela N Leal, Marco F Silva, Elisabeth G C Fernandes, Camila M P França, Fernando H C Souza, Clovis A Silva
Contraception is an important issue and should be a matter of concern in every medical visit of adolescent and young patients with chronic rheumatic diseases. This narrative review discusses contraception methods in adolescents with juvenile systemic lupus erythematosus (JSLE), antiphospholipid syndrome (APS), juvenile idiopathic arthritis (JIA) and juvenile dermatomyositis (JDM). Barrier methods are safe and their use should be encouraged for all adolescents with chronic rheumatic diseases. Combined oral contraceptives (COC) are strictly prohibited for JSLE and APS patients with positive antiphospholipid antibodies...
July 16, 2016: Revista Brasileira de Reumatologia
Satoru Teshigawara, Yoshinori Katada, Yuichi Maeda, Maiko Yoshimura, Eriko Kudo-Tanaka, Soichiro Tsuji, Yoshinori Harada, Masato Matsushita, Shiro Ohshima, Kotaro Watanabe, Takahiro Kumode, Yoshihiko Hoshida, Yukihiko Saeki
BACKGROUND: Hemophagocytic lymphohistiocytosis associated with autoimmune diseases is seen in patients with systemic juvenile idiopathic arthritis, adult-onset Still's disease, and systemic lupus erythematosus, whereas it is rarely seen in patients with dermatomyositis. In addition, central nervous system involvement with dermatomyositis is rare. To the best of our knowledge, this is the first case of hemophagocytic lymphohistiocytosis complicated by leukoencephalopathy in a patient with dermatomyositis accompanied with peripheral T-cell lymphoma...
2016: Journal of Medical Case Reports
E L Nasonov, T V Popkova, D S Novikova
The representatives of immunoinflammatory diseases are rheumatic ones, such as primarily rheumatoid arthritis, juvenile idiopathic arthritis, spondyloarthritis, psoriatic arthritis, systemic lupus erythematosus, and other systemic connective diseases, which are characterized by a high risk for untimely death. The high risk of untimely death in these diseases has been found to be associated with the severity of an immunoinflammatory process that gives rise to severe irreversible damage to vital organs and systems and with the development of a wide spectrum of comorbidities (infections, interstitial lung disease, malignant tumors, osteoporotic fractures, etc...
2016: Terapevticheskiĭ Arkhiv
Eve M D Smith, Michael W Beresford
Juvenile-onset systemic lupus erythematosus (JSLE) is a rare, severe multisystem autoimmune disease affecting the kidney (Lupus Nephritis, LN) in up to 80% of children. LN is more severe in children than adults, with potential for irreversible kidney damage requiring dialysis or transplant. Renal biopsy is currently the gold standard for diagnosing and monitoring LN, however, it is invasive and associated with complications. Urine biomarkers have been shown to be better than serum biomarkers in differentiating renal disease from other organ manifestations...
June 29, 2016: Clinical Immunology: the Official Journal of the Clinical Immunology Society
E D Batu, A Erden, E Seyhoğlu, L Kilic, Y Büyükasık, O Karadag, Y Bilginer, S A Bilgen, A Akdogan, S Kiraz, A I Ertenli, S Özen, U Kalyoncu
OBJECTIVES: Reactive haemophagocytic syndrome (RHS) is a hyperinflammatory disorder often occurring in the background of several disorders such as infections, malignancies, and rheumatic diseases. Recently, a score known as the HScore was developed for the diagnosis of RHS. In the original study, most of the patients had underlying haematological malignancy or infection and the best cut-off value for the HScore was 169 (sensitivity 93%; specificity 86%). In this study we aimed to analyse the performance of the HScore in rheumatic disease-related RHS...
June 30, 2016: Scandinavian Journal of Rheumatology
Rolando Cimaz
No abstract text is available yet for this article.
July 2016: Nature Reviews. Rheumatology
Siri Opsahl Hetlevik, Berit Flatø, Marite Rygg, Ellen Berit Nordal, Cathrine Brunborg, Helene Hetland, Vibke Lilleby
OBJECTIVES: To describe the characteristics, outcome and predictive factors of juvenile mixed connective tissue disease (JMCTD) in a nationwide cohort of patients. METHODS: We examined 55 patients with JMCTD after a mean disease duration of 16.2 years (SD 10.0). Patients were registered according to Kasukawa's criteria. Remission criteria were defined according to those for juvenile idiopathic arthritis, plus absence of cytopenia, myositis, progressive sclerodactyly, lung and oesophageal manifestations...
June 9, 2016: Annals of the Rheumatic Diseases
Priscilla S Freire, João D Montoni, Aline S M Ribeiro, Heloísa H Marques, Thais Mauad, Clovis A Silva
INTRODUCTION: One of the main issues in juvenile systemic lupus erythematosus (JSLE) patients is infection, such as tuberculosis (TB). Of note, SLE patients are susceptible to pulmonary and extrapulmonary TB. However, to our knowledge, this contagious disease was rarely reported in pediatric lupus population, particularly diffuse or miliary TB. Therefore, from January 1983 to December 2011, 5,635 patients were followed-up at our Pediatric Rheumatology Unit and 285 (5%) of them met the American College of Rheumatology classification criteria for SLE...
May 2016: Revista Brasileira de Reumatologia
Renan Bazuco Frittoli, Karina de Oliveira Peliçari, Bruna Siqueira Bellini, Roberto Marini, Paula Teixeira Fernandes, Simone Appenzeller
OBJECTIVE: To determine whether there is an association between the profile of cognitive dysfunction and academic outcomes in patients with juvenile systemic lupus erythematosus (JSLE). METHODS: Patients aged ≤18 years at the onset of the disease and education level at or above the fifth grade of elementary school were selected. Cognitive evaluation was performed according to the American College of Rheumatology (ACR) recommendations. Symptoms of anxiety and depression were assessed by Beck scales; disease activity was assessed by Systemic Lupus Erythematosus Disease Activity Index (SLEDAI); and cumulative damage was assessed by Systemic Lupus International Collaborating Clinics (SLICC)...
May 2016: Revista Brasileira de Reumatologia
Nádia E Aikawa, Ana P Nascimento, André L S Hayata, Eloisa Bonfá, Cláudia Goldenstein-Schainberg
OBJECTIVE: To evaluate the prevalence of hepatitis C virus (HCV) infection in high risk juvenile systemic lupus erythematosus (JSLE). STUDY DESIGN: Forty low income JSLE patients (6M:34F; mean age 19±4.4 yrs; mean disease duration 6±3.2 yrs) were studied. Twenty healthy children and adolescents matched for social economical level were included as controls. Anti-HCV tests were performed using a third generation microparticle enzyme immunoassay. Inclusion criterion was low social economical level...
May 2016: Revista Brasileira de Reumatologia
J M Lai, F Q Wu, Z X Zhou, Y C Yan, G X Su, S N Li, J Zhu, M Kang
OBJECTIVE: To investigate the value of brain magnetic resonance imaging (MRI) in evaluating the intracranial injuries in patients with juvenile onset systemic lupus erythematosus (SLE). METHOD: Data of brain MRI, CT, electroencephalogram (EEG), cerebrospinal fluids analysis and clinical features of the central nervous system of 44 patients from March 2007 to March 2015 with juvenile onset SLE who were not treated with glucocorticoids (Gcs) and immunosuppressive agents (Is) were retrospectively analyzed and compared...
June 2, 2016: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
S Harsini, V Ziaee, F Tahghighi, M Mahmoudi, A Rezaei, S Soltani, M Sadr, M H Moradinejad, Y Aghighi, N Rezaei
PURPOSE: Juvenile systemic lupus erythematosus (JSLE) is a severe and chronic autoimmune disease of unknown origin. Inflammatory cytokines can play a pivotal role in the pathogenesis of JSLE, while their secretion is under genetic control. The current investigation was performed to analyse the associations of particular single nucleotide polymorphisms (SNPs) of interleukin-2 (IL-2) and interferon-gamma (IFN-γ) genes in a case control study. MATERIALS AND METHODS: The allele, genotype and haplotype frequencies of the polymorphic IL-2 (G/T at -330, rs2069762, and G/T at +166, rs2069763) and IFN-γ (A/T at +874, rs2430561) genes were estimated in 59 patients with JSLE by contrast with 140 healthy controls using polymerase chain reaction with sequence-specific primers method...
September 2016: Allergologia et Immunopathologia
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